THERAPEUTICS

Brands

• Syprine

Generic?

• Yes

Class

• Chelating agent

Commonly Prescribed for

(FDA approved in bold)

• Wilson's disease (WD) in patients intolerant of penicillamine

How the Drug Works

• In WD copper accumulates in body tissues (especially the liver and CNS), causing neurological/psychiatric problems and/or liver failure. Trientine binds to (chelates) copper, allowing it to be excreted in the urine

How Long Until It Works

• 6 months or more

If It Works

• Continue treatment, if tolerated. Most patients remain on drug for the rest of their life but if serum copper returns to normal (< 10mcg/dL) consider changing to elemental zinc or zinc sulfate. Monitor for recurrence of symptoms or changes in urinary copper excretion

If It Doesn't Work

• Increase to as much as 2000mg daily for poor clinical response or if free serum copper is above 20mcg/dL. For liver failure or truly refractory patients, liver transplantation is curative

Best Augmenting Combos for Partial Response or Treatment-Resistance

• Change to penicillamine if ineffective. A diet low in copper-containing foods, such as nuts, chocolate, liver, and dried fruit, is recommended

Tests

• Adequately treated patients should have free serum copper below 10mcg/dL. Monitor 24-hour urinary copper excretion every 6–12 months (should be between 0.5 and 1mg)

ADVERSE EFFECTS (AEs)

How the Drug Causes AEs

• Unknown

Notable AEs

• Heartburn, iron deficiency anemia, anorexia, cramps, muscle pain, and epigastric pain have been reported. Rarely muscle spasm or dystonia has occurred. The relationship of these symptoms to trientine is unclear

Life-Threatening or Dangerous AEs

• Myasthenia gravis and systemic lupus erythematosus have been reported

Weight Gain

• Unusual

Sedation

• Unusual

What to Do About AEs

• Discontinue only for serious AEs

Best Augmenting Agents to Reduce AEs

• Most AEs cannot be reduced with the use of an augmenting agent

DOSING AND USE

Usual Dosage Range

• 1000–2000mg/day

Dosage Forms

• Tablets: 250mg